Thread by @RosenelliEM

It's time for a slightly tardy #SpacedRepetition: @CPSolvers

Chat recap of the #ClinicalPearls #VirtualMorningReport

July 10th Day 91: clinicalproblemsolving.com/morning-report…
w/ @AnnKumfer @DxRxEdu @rabihmgeha
Teaching points: @sukritibanthiya

Get ready for the @fernandbteich show

But first, we want to acknowledge @_HarryPaul_ who gave us all something to reflect on. #MichaelHickson
washingtonpost.com/health/2020/07…

Thanks for stepping up and sharing Harry!

Let’s start with a problem representation:

A 69-y/o F w/ a PMH of sinus cancer presents from an outside hospital with night sweats that started 4 wks ago along w/ rigors, fevers, nausea, early satiety, & weightloss found to have splenomegaly, lymphopenia, & elevated ESR/CRP

Given the history of sinus cancer, did she have any radiation therapy?

We want to know if she is currently immunosuppressed?

That is a big portal of entry for various local/disseminated infection

Her location Missouri is full of fungi

Rigors are predictors of bacteremia - chills without rigors are not

Now we have some more info: subacute-chronic, progressive cough, constitutional symptoms, dysphagia, early satiety, asthenia vs weakness in background of SE USA travel and multiple courses of abx

her current inflammatory syndrome does not localize anywhere

While in the hospital she got a ton of abx without avail, doxy and cextriaxone, broadened to vanc, mero, and amp B

But now we here she likes cave spelunking, hmm

histo loves it some caves, ncbi.nlm.nih.gov/m/pubmed/37465…

let's be systematic. this could be a subacute infection (atypical bacteria/fungi/parasites/viruses), a cancer or paraneoplastic syndrome related to a cancer or an autoimmune disease

if it's autoimmune we dont have enough localizing features to define it

not to leave the infection bucket, but in the back of my mind, I'm working with radiation to the head/neck and weakness/early satiety...wondering about a central endocrinopathy

in terms of infection, she does not seem immunocompromised at least based on her story

so this leaves me with atypical mycobacteria, TB, endemic fungi, parasites less likely in the midwest

viruses: a generic EBV-CMV-HIV infection can cause this
and may not respond to any antibiotics

Remember drugs can cause inflammatory syndromes but she is on nothing special

if this is SCC of the head and neck they're generally bland in terms of paraneoplastic syndromes except for PTHrP hyperCa

does she have nocardiosis and needs TMP/SMX? hmmmm, ncbi.nlm.nih.gov/pmc/articles/P…

Adding the splenomegaly to the picture:

splenomegaly w/ inflammation --> a new cancer? (Myeloproliferative disorder? lymphoma marginal zone/follicular/DLBCL?), new infection?

Are there non-cancer cause splenomegaly other than Mono? What about leishmaniasis or erlichiosis

Also mono syndromes, babesiosis, leishmania, malaria, salmonella

Can we use the presence vs absence of splenomegaly to determine the tempo of the splenomegaly manifestation?ncbi.nlm.nih.gov/books/NBK43090…

Malaria would be unlikely this far out from her residence in SE Asia.

what was the infection prevalent in east asia?

meliodosis from burkholderia pseudomallei, cdc.gov/melioidosis/in…

Now add her normal WBC but lymphopenia:

With neg BCx, unlikely to be meliodosis. Also meliodosis patients tend to present extremely sick

Her spleen is radpidly enlarging, something is growing there rapidly

is this an aggressive lymphoma?

is this burkitt's?

is this DLBCL?

Lets give an updated PR: 69yo F w/ hx of (remote?) sinus cancer s/p surgery + RT coming with subacute inflammation, rapidly progressing splenomegaly and mild liver injury.

Has so may B symptoms, but only splenomegaly. Could consider lymphoma without LAD = intravascular lymphoma

something in the spleen is spitting interleukins++ in the blood

doesn’t seem like hemolysis given the nml direct bili, but can think about babesia as well, the American malaria, cdc.gov/ticks/tickborn…
Babesia looks almost identical to malaria on smear, & treated w/ similarly

Solid cancers are allergic to spleen,

Her increased ferritin = I have inflammation

Still's doesn't have the lipid component of HLH & there is no rash or joint pain

anything that over activates the immune system can cause some degree of "HLH"

HLH is the “Lupus” of VMR

Fernand is the pearl dropper and expert investigator we all need in our lives

Quick HScore puts 40-54% probability of HLH even without positive bone marrow biopsy

And the final dx is.....

Primary Splenic Diffuse Large B-Cell Lymphoma (DLBCL) localized to a single organ
ncbi.nlm.nih.gov/pmc/articles/P…

is very rare as it occurs in less than 1% of non-Hodgkin’s lymphomas

It is most commonly found in females and older males

Symptoms can include splenomegaly as well as left upper quadrant pain, fever and weight loss.

It can also be associated with human immunodeficiency virus (HIV), and can present with metastasis to hilar and retroperitoneal lymph nodes"

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